Introduction: Kawasaki disease (KD) is the most common cause of childhood vasculitis affecting small- and medium-sized arteries. The cause of this disease is unknown with an overall prevalence of 0.10% making this a rare entity. Case presentation: Here the authors present an index case of a 2-year-old child presenting with a persistent high-grade fever of more than 5 days and a 3-day history of bilateral swelling of hands and feet along with cervical lymphadenopathy. On the subsequent day of admission, the child developed mucocutaneous symptoms and cervical lymphadenopathy. The diagnosis of KD was made, and it was successfully treated with intravenous immunoglobulin and aspirin. Clinical discussion: Timely diagnosis and early treatment of KD are challenging due to the lack of definitive diagnostic tests. Watchful waiting for symptoms may be necessary before a diagnosis can be made because not all clinical symptoms are present simultaneously as in the index case. Conclusions: This case highlights considering KD as a differential diagnosis of nonresolving fever in children with mucocutaneous findings. Intravenous immunoglobulin along with aspirin is the mainstay of therapy and should be started as early as possible to prevent detrimental cardiac complications. There is a high tendency of diagnostic dilemmas due to a wide array of nonspecific presentations thus healthcare providers must be more vigilant of this entity.

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